Spinal Hemangioblastomas: Clinical Presentation, Radiology, and Treatment

Abstract

tion (WHO) grade I benign tumors that occur throughout the neuraxis. Spinal hemangioblastomas (SHs) make up 2% to 15% of all spinal intramedullary tumors. These highly vascular tumors usually arise from the dorsolateral pia mater. Considerable progress has been made in the surgical treatment of SHs since the fi rst report of successful excision by Schultze in 1912. The principles for surgical resection of SHs are similar to those for the arteriovenous malformations (AVMs) and different from the intramedullary glial tumors. Both SH and AVM require a circumferential dissection from the surrounding normal tissue, whereas in contrast to the intramedullary glial tumors, resection of SH and AVM is performed en bloc without internal debulking, because of the highly vascular nature of these tumors. Epidemiology SH is the third most frequently found intramedullary spinal cord tumor, after gliomas and ependymomas. They commonly occur in young adults age 30 to 40 years, with some patients having a positive family history. Hemangioblastomas are more common in the posterior fossa than the spinal cord. SH can arise sporadically (∼60% of cases) or in the context of von Hippel-Lindau (VHL) disease. Sporadic cases are generally in patients who are a decade older than those with VHL disease. Multiple tumors are observed in 60% to 80% of VHL-associated hemangioblastomas, and most are revealed as smaller lesions. In contrast, multifocal tumors comprise only about 3% of sporadic hemangioblastomas.